Abstract
Six children from 2 to 13 years of age with SS were treated with ETX for the following serious complications: pneumococcal sepsis and meningitis, hepatic crisis, severe hepatic dysfunction before cholecystectomy, SS lung syndrome, SS lung syndrome and congestive heart failure and refractory painful crisis. Hemoglobin S ranged from 64.6 to 100% with a mean of 86.7% pre-ETX compared to 21 to 33% with a mean of 27.6% post-exchange. Relief of symptoms and improvement in the functions of affected organs were observed in all patients following ETX. In the hepatic crisis the total bllirubin level of 28.5 mg/dl (15.0 mg/dl direct) prior to ETX decreased to 6.9 mg/dl (2.4 mg/dl direct). In the SS lung syndrome the PO2 was 40 mm Hg prior to and 99 mm Hg after ETX. Technically the procedure of ETX is easy. ETX effectively removes irreversibly sickled cells, reduces the viscosity of blood and improves the micro-circulation without volume overload which would be the case in simple transfusion. For these reasons ETX merits use in potentially fatal or chronically debilitating complications or in the preparation for surgery in selected cases of SS.
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Shende, A., Karayalcin, G. & Lanzkowsky, P. THE USE OF PARTIAL EXCHANGE TRANSFUSION (ETX) IN CHILDREN WITH SERIOUS COMPLICATIONS OF SICKLE CELL ANEMIA (SS). Pediatr Res 11, 480 (1977). https://doi.org/10.1203/00006450-197704000-00661
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DOI: https://doi.org/10.1203/00006450-197704000-00661